{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T15:40:02Z","timestamp":1745768402238,"version":"3.40.4"},"reference-count":25,"publisher":"BMJ","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["BMJ Case Reports"],"abstract":"<jats:p>A 55-year-old alcoholic man presented with firm hepatomegaly, ascites and markedly elevated alkaline phosphatase. He had a history of pulmonary tuberculosis. Work-up for malignancy was negative. Histological examination of liver showed extracellular deposition of pink amorphous material which is Congo red stain negative. Deteriorating renal function and nephrotic-range proteinuria were noted. Renal histology showed thickening of the glomerular and tubular basement membranes by non-congophilic deposits along with mesangial expansion. Bone marrow examination revealed patchy areas of pink amorphous deposits which are Congo red stain negative. Immunohistochemical staining of amorphous depositions in liver, kidney and bone marrow were positive for \u03ba light chains. Serum-free light chain assay confirmed markedly elevated free \u03ba-light chain. \u03ba-light chain deposition disease is a systemic disease with universal renal involvement but rarely it presents as chronic cholestatic liver disease with portal hypertension and frequently associated with fatal outcome due to diagnostic delay.<\/jats:p>","DOI":"10.1136\/bcr-2013-009553","type":"journal-article","created":{"date-parts":[[2013,5,30]],"date-time":"2013-05-30T04:05:01Z","timestamp":1369886701000},"page":"bcr2013009553","source":"Crossref","is-referenced-by-count":2,"title":["Portal hypertension related to light chain deposition disease of liver: an enlightening experience"],"prefix":"10.1136","volume":"2013","author":[{"given":"Arunansu","family":"Talukdar","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kabita","family":"Mukherjee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dibbendhu","family":"Khanra","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manjari","family":"Saha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2013,5,29]]},"reference":[{"key":"2025042708064484000_2013.may29_1.bcr2013009553.1","doi-asserted-by":"crossref","first-page":"455","DOI":"10.7326\/0003-4819-76-3-112-6-455","article-title":"Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition disease and their relation to light chain amyloidosis","volume":"2013","author":"Buxbaum","year":"1990","journal-title":"Ann Intern Med"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.2","first-page":"545","article-title":"Synthesis of abnormal heavy and light chains in multiple myeloma with visceral deposition of mono-clonal immunoglobulin","volume":"2013","author":"Preud'homme","year":"1980","journal-title":"Clin Exp Immunol"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.3","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1056\/NEJM199311043291905","article-title":"Brief report: heavy-chain deposition disease","volume":"2013","author":"Aucouturier","year":"1993","journal-title":"N Engl J Med"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.4","first-page":"370A","article-title":"Light chain deposits in multiple myeloma","volume":"2013","author":"Antonovych","year":"1974","journal-title":"Lab Invest"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.5","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/0002-9343(76)90440-X","article-title":"Manifestations of systemic light chain deposition","volume":"2013","author":"Randall","year":"1976","journal-title":"Am J Med"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.6","doi-asserted-by":"crossref","first-page":"1154","DOI":"10.1053\/j.ajkd.2003.08.040","article-title":"Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors","volume":"2013","author":"Pozzi","year":"2003","journal-title":"Am J Kidney Dis"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.7","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1053\/snep.2002.33673","article-title":"Kidney and liver involvement in monoclonal light chain disorders","volume":"2013","author":"Pozzi","year":"2002","journal-title":"Semin Nephrol"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.8","first-page":"527","article-title":"Spectrum of glomerular and tubule-interstitial renal lesions associated with monotypical immunoglobulin light chain deposition","volume":"2013","author":"Sanders","year":"1991","journal-title":"Lab Invest"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.9","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1681\/ASN.V1271558","article-title":"Light chain deposition disease: a model of glomerulosclerosis defined at the molecular level","volume":"2013","author":"Ronco","year":"2001","journal-title":"J Am Soc Nephrol"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.10","doi-asserted-by":"crossref","first-page":"307","DOI":"10.3109\/00313021003631171","article-title":"Light chain deposition disease presenting as massive hepatomegaly","volume":"2013","author":"Nath","year":"2010","journal-title":"Pathology"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.11","first-page":"429","article-title":"Light chain deposition disease of the liver","volume":"2013","author":"Gireli","year":"1999","journal-title":"Eur J Gastroenterol Hepatol"},{"key":"2025042708064484000_2013.may29_1.bcr2013009553.12","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/0168-8278(91)90913-V","article-title":"Light chain deposition disease of the liver associated with AL-type amyloidosis and severe cholesta-sis. 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